There is limited data on the long-term efficacy of intravenous
iloprost in patients with
pulmonary arterial hypertension (PAH). This retrospective multicentre analysis evaluated the
clinical course of patients with PAH treated with i.v.
iloprost, in most cases after having received inhaled
iloprost as first-line
therapy. Between 1997 and 2001, 79 PAH patients were treated with i.v.
iloprost and followed until 2007. These patients had advanced and progressive disease as indicated by a mean pulmonary vascular resistance of 1,533 dyn x s x cm(-5) at the time of diagnosis and of 1,858 dyn x s x cm(-5) at the onset of i.v.
iloprost therapy. Introduction of i.v.
iloprost therapy resulted in initial haemodynamic and clinical improvement. At the end of the observation period, however, 50 (61%) patients had died and 21 (26%) required
lung transplantation.
Transplantation-free survival rates at 1, 3, and 5 yrs were 86%, 59% and 45%, respectively, after the diagnosis of PAH, and 54%, 31% and 15%, respectively, after the introduction of i.v.
iloprost therapy. Predictors of an adverse outcome at baseline were a low 6-min walk distance and a low mixed venous oxygen saturation. In conclusion, despite initial haemodynamic and clinical improvement, overall long-term survival with i.v.
iloprost therapy was limited.